Gustatory Function in Patients With Cerebellopontine Angle Masses.

OBJECTIVE: To investigate the impact of cerebellopontine angle (CPA) masses on subjective and measured taste function. STUDY DESIGN: Retrospective cross-sectional study. SETTING: Tertiary referral center. PATIENTS: Consecutive adult patients with untreated CPA masses. INTERVENTIONS: Gustatory function was psychophysically measured with Taste Strips (range, 0-16) on both sides of the tongue. Subjective taste complaints were assessed using a questionnaire. MAIN OUTCOME MEASURES: Half-sided taste impairment (hemi-ageusia) was defined as side-to-side asymmetry ≥4 points with <9 points on the side of the CPA mass. We used the Koos classification for vestibular schwannomas (VS) and, in the case of facial nerve palsy, the House-Brackmann grading system. RESULTS: We included 135 patients (mean [standard deviation (SD)] age, 55.3 ± 14.1 yr; 62 males). The most common CPA mass was VS (77%). Overall, the measured taste function was lower on the affected compared with the healthy side of the tongue (mean score, 9.8 ± 3.3 versus 11 ± 2.9; p < 0.0001). Looking for clinically relevant one-sided taste impairment revealed 18 (13.3%) patients with hemi-ageusia, but only 4 (30.8%) of those subjectively complained of taste dysfunction. Regarding VS, Koos IV masses presented the lowest score on the affected side (mean score, 7.5 ± 3.7). Six patients presented with facial palsy. Having facial palsy did not result in a lower Taste Strips score (p = 0.23). CONCLUSION: Before any CPA mass treatment, a measurable ipsilateral decrease in gustatory function is present in many patients. Most patients do not notice this preexisting taste impairment. From a medicolegal standpoint, this warrants consideration. To avoid postoperative claims regarding taste function, a preoperative assessment may be considered.

[Management of patients with complex ear deformities]. / Prise en charge des patients présentant des déformations complexes du pavillon de l'oreille.

Complex ear reconstruction requires specialized multidisciplinary care. Most patients present with microtia, often associated with hearing disorders. The management of these disorders is a priority, and reconstruction of the external ear remains optional. Nowadays, auricular reconstruction is based on the subcutaneous implantation of either autologous cartilage or an allogeneic implant. Autologous reconstruction requires highly specialized surgical expertise and involves harvesting rib cartilage but carries a lower risk of exposure compared to allogeneic implants. Both techniques yield good results with a high success rate and have a positive impact on the social functioning and daily life of patients.

La reconstruction complexe du pavillon auriculaire nécessite une prise en charge multidisciplinaire spécialisée. La majorité des patients nécessitant ce geste présentent une microtie, souvent associée à des troubles de l'audition. La prise en charge de ceux-ci est prioritaire et la reconstruction du pavillon reste facultative. Aujourd'hui, la reconstruction du pavillon se base sur l'implantation sous-cutanée d'une maquette de cartilage autologue ou d'un implant allogène. La reconstruction autologue demande une expertise chirurgicale hautement spécialisée et nécessite un prélèvement de cartilage costal mais présente un risque d'exposition inférieur par rapport à l'implant allogène. Les deux techniques permettent d'atteindre de bons résultats avec un taux de réussite élevé et un effet positif sur le fonctionnement social et le quotidien des patients.

Optimizing vestibular implant electrode positioning using fluoroscopy and intraoperative CT imaging.

PURPOSE:  Vestibular implant electrode positioning close to the afferent nerve fibers is considered to be key for effective and selective electrical stimulation. However, accurate positioning of vestibular implant electrodes inside the semicircular canal ampullae is challenging due to the inability to visualize the target during the surgical procedure. This study investigates the accuracy of a new surgical protocol with real-time fluoroscopy and intraoperative CT imaging, which facilitates electrode positioning during vestibular implant surgery. METHODS:  Single-center case-controlled cohort study with a historic control group at a tertiary referral center. Patients were implanted with a vestibulocochlear implant, using a combination of intraoperative fluoroscopy and cone beam CT imaging. The control group consisted of five patients who were previously implanted with the former implant prototype, without the use of intraoperative imaging. Electrode positioning was analyzed postoperatively with a high-resolution CT scan using 3D slicer software. The result was defined as accurate if the electrode position was within 1.5 mm of the center of the ampulla. RESULTS: With the new imaging protocol, all electrodes could be positioned within a 1.5 mm range of the center of the ampulla. The accuracy was significantly higher in the study group with intraoperative imaging (21/21 electrodes) compared to the control group without intraoperative imaging (10/15 electrodes), (p = 0.008). CONCLUSION:  The combined use of intraoperative fluoroscopy and CT imaging during vestibular implantation can improve the accuracy of electrode positioning. This might lead to better vestibular implant performance.

[Malignant external auditory canal tumors : management of a rare tumor]. / Tumeurs malignes du conduit auditif externe : prise en charge d'une tumeur rare.

Malignant tumors of the external auditory canal are rare tumors and very often diagnosed at an advanced stage due to non-specific symptoms. The best treatment is the radical surgery, eventually followed by radiotherapy. A multidisciplinary team is essential to realize an optimal management. Prognosis remains unfavorable for advanced tumors.

Les tumeurs malignes du conduit auditif externe (CAE) sont rares et leur diagnostic est souvent retardé en raison de symptômes peu spécifiques. Le traitement de choix consiste en une chirurgie radicale, éventuellement suivie d'une radiothérapie. Une équipe multidisciplinaire est indispensable pour une prise en charge optimale. Le pronostic reste défavorable dans les tumeurs avancées.

Increased Petrous Bone Uptake on 18 F-PSMA-1007 PET/CT Due to Otospongiosis.

ABSTRACT: We report the case of a 74-year-old man who had undergone radical prostatectomy for prostatic cancer 6 months earlier. Elevated prostate-specific antigen during follow-up prompted 18 F-prostate-specific membrane antigen (PSMA) ligand PET/CT ( 18 F-PSMA-1007 PET/CT) to search for new manifestations of prostate cancer, revealing an increased focal uptake (SUV max , 5.9) in the left cochlear/pericochlear temporal bone and equivocal PSMA-RADS-3a external iliac nodes. Comparison with cone-beam CT and MRI showed that the focal temporal bone uptake corresponded to the typical morphological features of active otospongiosis (otosclerosis) in the context of a previously known long-standing otospongiosis.

Diagnostic value and prognostic significance of MRI findings in sudden sensorineural hearing loss.

Purpose: We evaluated the clinical significance of magnetic resonance imaging (MRI) findings and their prognostic value for initial hearing loss and recovery in patients with sudden sensorineural hearing loss (SSNHL). Materials and Methods: This retrospective study included consecutive adult patients with unilateral SSNHL, contrast-enhanced MRI and audiometric testing evaluated in our institution between 2005 and 2017. MRI reports, patient data, treatment, and audiometric tests were reviewed, with the relationship between MRI findings and hearing loss/recovery analyzed. Results: Overall, 266 patients were included. Additional symptoms comprised tinnitus (114/266; 43%), vertigo (45/266; 17%), ear pain (26/266; 10%), and ear pressure (6/266; 2%). At least one cardiovascular risk factor (hypertension, diabetes, hypercholesterolemia, cardiopathy, and active smoking) existed in 167/266 (63%) patients. Corticosteroid treatment was followed by 198/266 (74%) patients while contraindications/refusal/compliance precluded treatment in 68/266(26%). Complete, partial or slight hearing recovery occurred in 167/266 (63%) patients. Three MRI patient groups were identified: a group with normal MRI examinations or incidentalomas (128/266; 48%), a group with peripheral auditory system (PAS) lesions (95/266; 36%), and a group with central nervous system (CNS) lesions (43/266; 16%). PAS lesions included lesions from the cochlea to the brain stem (e.g., schwannoma, meningioma, labyrinthitis, intracochlear hemorrhage, vestibulocochlear neuritis), whereas CNS lesions corresponded in 42/43(98%) of cases to leukoaraiosis and other vascular lesions (e.g., stroke, hemorrhage, aneurysm, venous sinus thrombosis, and cavernoma). Belonging to one of the three MRI groups did not influence the degree of initial hearing loss, affected frequencies or treatment, p > .05. Gender and cardiovascular risk factors did neither affect initial hearing loss nor recovery. However, age > 70 years negatively affected initial hearing loss in all frequencies, as well as recovery in all frequencies except 1000 Hz. Also, poor recovery of initial high-frequency hearing loss (>1000 Hz) was significantly associated with CNS lesions. Conclusion: Age > 70 years and CNS lesions depicted by MRI independently predicted poor auditory recovery, albeit in different frequencies. Lay Summary: In patients with sudden hearing loss, older age (above 70 years) predicts poorer hearing recovery than in younger patients in most hearing frequencies. In addition, abnormalities of brain tissue revealed by MRI predict poorer hearing recovery at high frequencies. Level of Evidence: Level III.

Sound localization in patients with bilateral vestibulopathy.

PURPOSE: The goal of this study was to evaluate if bilaterally (partially) absent vestibular function during static sound localization testing, would have a negative impact on sound localization skills. Therefore, this study compared horizontal static sound localization skills of normal-hearing patients with bilateral vestibulopathy (BV) and healthy controls. METHODS: Thirteen normal-hearing patients with BV and thirteen age-matched healthy controls were included. Sound localization skills were tested using seven loudspeakers in a frontal semicircle, ranging from - 90° to + 90°. Sound location accuracy was analyzed using the root-mean-square error (RMSE) and the mean absolute error (MAE). To evaluate the severity of the BV symptoms, the following questionnaires were used: Dizziness Handicap Inventory (DHI), Oscillopsia severity questionnaire (OSQ), 12-item Spatial, Speech, and Qualities Questionnaire (SSQ12), and Health Utilities Index Mark 3 (HUI3). RESULTS: The RMSE and MAE were significantly larger (worse) in the BV group than in the healthy control group, with respective median RMSE of 4.6° and 0°, and a median MAE of 0.7° and 0°. The subjective reporting of speech perception, spatial hearing, and quality of life only demonstrated a moderate correlation between DHI (positive correlation) and HUI total score (negative correlation), and localization scores. CONCLUSION: Static sound localization skills of patients with BV were only mildly worse compared to healthy controls. However, this difference was very small and therefore most likely due to impaired cognitive function. The vestibular system does not seem to have a modulating role in sound localization during static conditions, and its impact is negligible in contrast to the impact of hearing impairment. Furthermore, the subjective reporting of speech perception, spatial hearing, and quality of life was not strongly correlated with localization scores.

Upbeat vertical nystagmus after brain stem cavernoma resection: a rare case of nucleus intercalatus/nucleus of roller injury.

INTRODUCTION: CNS cavernomas are a type of raspberry-shaped vascular malformations that are typically asymptomatic, but can result in haemorrhage, neurological injury, and seizures. Here, we present a rare case of a brainstem cavernoma that was surgically resected whereafter an upbeat nystagmus presented postoperatively. CASE REPORT: A 42-year old man presented with sudden-onset nausea, vomiting, vertigo, blurred vision, marked imbalance and difficulty swallowing. Neurological evaluation showed bilateral ataxia, generalized hyperreflexia with left-sided predominance, predominantly horizontal gaze evoked nystagmus on right and left gaze, slight left labial asymmetry, uvula deviation to the right, and tongue deviation to the left. MRI demonstrated a 13-mm cavernoma with haemorrhage and oedema in the medulla oblongata. Surgery was performed via a minimal-invasive, midline approach. Complete excision was confirmed on postoperative MRI. The patient recovered well and became almost neurologically intact. However, he complained of mainly vertical oscillopsia. The videonystagmography revealed a new-onset spontaneous upbeat nystagmus in all gaze directions, not suppressed by fixation. An injury of the rarely described intercalatus nucleus/nucleus of Roller is thought to be the cause. CONCLUSION: Upbeat nystagmus can be related to several lesions of the brainstem, including the medial longitudinal fasciculus, the pons, and the dorsal medulla. To our knowledge, this is the first case of an iatrogenic lesion of the nucleus intercalatus/nucleus of Roller resulting in an upbeat vertical nystagmus. For neurologists, it is important to be aware of the function of this nucleus for assessment of clinical manifestations due to lesions within this region.

Prospective cohort study on the predictors of fall risk in 119 patients with bilateral vestibulopathy.

OBJECTIVES: To identify predictive factors for falls in patients with bilateral vestibulopathy (BV). Specific variables contributing to the general work-up of a vestibular patient were compared between BV patients experiencing falls and those who did not. DESIGN: Prospective multi-centric cohort study. SETTING: Department of Otorhinolaryngology & Head and Neck Surgery at two tertiary referral centers: Antwerp University Hospital and Maastricht University Medical Center. PARTICIPANTS: In total, 119 BV patients were included. BV diagnosis was defined in accordance with the diagnostic BV criteria, established by the Bárány Society in 2017. MAIN OUTCOME MEASURES: Patients were divided into fallers and non-fallers, depending on the experience of one or more falls in the preceding 12 months. Residual vestibular function on caloric testing, rotatory chair testing, video head impulse test (vHIT) and cervical vestibular evoked myogenic potentials (cVEMP) was evaluated as a predictive factor for falls. Furthermore, hearing function (speech perception in noise (SPIN)), sound localization performance, etiology, disease duration, sport practice, scores on the Dizziness Handicap Inventory (DHI) and the Oscillopsia Severity Questionnaire (OSQ) were compared between fallers and non-fallers. RESULTS: Forty-five (39%) patients reported falls. In a sub-analysis in the patients recruited at UZA (n = 69), 20% experienced three or more falls and three patients (4%) suffered from severe fall-related injuries. The DHI score and the OSQ score were significantly higher in fallers. Residual vestibular function, SPIN, sound localization performance, etiology, disease duration, age and sport practice did not differ between fallers and non-fallers. CONCLUSIONS: Falls and (severe) fall-related injuries are frequent among BV patients. A DHI score > 47 and an OSQ score > 27.5 might be indicative for BV patients at risk for falls, with a sensitivity of 70% and specificity of 60%. Residual vestibular function captured by single vestibular tests (vHIT, calorics, rotatory chair, cVEMP) or by overall vestibular function defined as the number of impaired vestibular sensors are not suitable to distinguish fallers and non-fallers in a BV population.

Drafting a Surgical Procedure Using a Computational Anatomy Driven Approach for Precise, Robust, and Safe Vestibular Neuroprosthesis Placement-When One Size Does Not Fit All.

OBJECTIVE: To design and evaluate a new vestibular implant and surgical procedure that should reach correct electrode placement in 95% of patients in silico. DESIGN: Computational anatomy driven implant and surgery design study. SETTING: Tertiary referral center. PARTICIPANTS: The population comprised 81 patients that had undergone a CT scan of the Mastoid region in the Maastricht University Medical Center. The population was subdivided in a vestibular implant eligible group (28) and a control group (53) without known vestibular loss. INTERVENTIONS: Canal lengths and relationships between landmarks were calculated for every patient. The relationships in group-anatomy were used to model a fenestration site on all three semicircular canals. Each patient's simulated individual distance from the fenestration site to the ampulla was calculated and compared with the populations average to determine if placement would be successful. MAIN OUTCOME MEASURES: Lengths of the semicircular canals, distances from fenestration site to ampulla (intralabyrinthine electrode length), and rate of successful electrode placement (robustness). RESULTS: The canal lengths for the lateral, posterior, and superior canal were respectively 12.1 mm ±â€Š1.07, 18.8 mm ±â€Š1.62, and 17.5 mm ±â€Š1.23, the distances from electrode fenestration site to the ampulla were respectively 3.73 mm ±â€Š0.53, 9.02 mm ±â€Š0.90, and 5.31 mm ±â€Š0.73 and electrode insertions were successful for each respective semicircular canal in 92.6%, 66.7%, and 86.4% of insertions in silico. The implant electrode was subsequently revised to include two more electrodes per lead, resulting in a robustness of 100%. CONCLUSIONS: The computational anatomy approach can be used to design and test surgical procedures. With small changes in electrode design, the proposed surgical procedure's target robustness was reached.

The Vestibular Implant: Hearing Preservation during Intralabyrinthine Electrode Insertion-A Case Report.

OBJECTIVE: The vestibular implant seems feasible as a clinically useful device in the near future. However, hearing preservation during intralabyrinthine implantation remains a challenge. It should be preserved to be able to treat patients with bilateral vestibulopathy and (partially) intact hearing. This case study investigated the feasibility of hearing preservation during the acute phase after electrode insertion in the semicircular canals. METHODS: A 40-year-old woman with normal hearing underwent a translabyrinthine approach for a vestibular schwannoma Koos Grade IV. Hearing was monitored using auditory brainstem response audiometry (ABR). ABR signals were recorded synchronously to video recordings of the surgery. Following the principles of soft surgery, a conventional dummy electrode was inserted in the lateral semicircular canal for several minutes and subsequently removed. The same procedure was then applied for the posterior canal. Finally, the labyrinthectomy was completed, and the schwannoma was removed. RESULTS: Surgery was performed without complications. No leakage of endolymph and no significant reduction of ABR response were observed during insertion and after removal of the electrodes from the semicircular canals, indicting no damage to the peripheral auditory function. The ABR response significantly changed when the semicircular canals were completely opened during the labyrinthectomy. This was indicated by a change in the morphology and latency of peak V of the ABR signal. CONCLUSION: Electrode insertion in the semicircular canals is possible without acutely damaging the peripheral auditory function measured with ABR, as shown in this proof-of-principle clinical investigation.

Visual acuity while walking and oscillopsia severity in healthy subjects and patients with unilateral and bilateral vestibular function loss.

OBJECTIVES: To assess visual acuity (VA) while the patient is walking and to evaluate oscillopsia severity in patients with bilateral vestibulopathy (BV) and in patients with unilateral vestibular loss (UVL). DESIGN: Prospective study with a group of patients with BV, a group of patients with UVL, and a control group of healthy subjects. SETTING: Tertiary academic center. PARTICIPANTS: Thirty seven patients with BV(age range, 29-80 years), 11 patients with UVL (age range, 48-75 years), and 57 healthy subjects (age range 20-77 years). INTERVENTION: Computation of the difference between the VA measured in static conditions and in dynamic conditions while walking on a treadmill at 2, 4, and 6 km/h. Oscillopsia severity was assessed with a questionnaire that we developed. MAIN OUTCOME MEASURES: Differences in VA at 2, 4, and 6 km/h and oscillopsia severity score. RESULTS: As a group, patients with BV showed a significant increase of the VA differences compared with healthy subjects (P < .001) and patients with UVL (P < .001) for all 3 walking velocities. Normality thresholds were defined as healthy subjects' 95% CI. Sensitivity of the test was 97% for discriminating patients with BV. Moderate to extreme oscillopsia severity was found in 81% of patients with BV and in 9% of patients with UVL. Differences in VA did not correlate with oscillopsia severity scores in patients with BV (P > .05 for all comparisons). CONCLUSIONS: We designed a highly sensitive, simple, cost-effective protocol to assess dynamic VA under physiologic conditions and a questionnaire to determine oscillopsia severity. Both tools could be used for the evaluation of new treatments for BV and patients with UVL.

Taste disorders after tonsillectomy: a long-term follow-up.

In a former study, taste disturbances after tonsillectomy seemed to be more frequent than expected. Eight percent of patients reported subjective taste disorders 6 months after tonsillectomy. Fifteen patients from the initial trial, who reported taste disorders after tonsillectomy, were contacted again for this long-term follow-up. A telephone interview using the same questionnaire addressing the current self-estimate of taste function was performed. At 32 ± 10 months following surgery, two (0.9%) patients still reported suffering from taste disturbance. This long-term follow-up study shows that dysgeusia following tonsillectomy occurs in approximately 1% of patients. These data should be considered when patients are informed about complications after tonsillectomy.

The modified ampullar approach for vestibular implant surgery: feasibility and its first application in a human with a long-term vestibular loss.

OBJECTIVE: To assess, for the first time in a human with a long-term vestibular loss, a modified approach to the ampullae and the feasibility of evoking a VOR by ampullar stimulation. MATERIALS AND METHODS: Peroperative stimulation of the ampullae, using the ampullar approach, was performed under full anesthesia during cochlear implantation in a 21-year-old female patient, who had experienced bilateral vestibular areflexia and sensorineural hearing loss for almost 20 years. RESULTS: The modified ampullar approach was performed successfully with as minimally invasive surgery as possible. Ampullar stimulation evoked eye movements containing vectors congruent with the stimulated canal. As expected, the preliminary electrophysiological data were influenced by the general anesthesia, which resulted in current spread and reduced maximum amplitudes of eye movement. Nevertheless, they confirm the feasibility of ampullar stimulation. CONCLUSION: The modified ampullar approach provides safe access to the ampullae using as minimally invasive surgery as possible. For the first time in a human with long-term bilateral vestibular areflexia, it is shown that the VOR can be evoked by ampullar stimulation, even when there has been no vestibular function for almost 20 years. This approach should be considered in vestibular surgery, as it provides safe access to one of the most favorable stimulus locations for development of a vestibular implant.

Taste disturbance following tonsillectomy--a prospective study.

OBJECTIVES/HYPOTHESIS: Persistent taste disturbance is a rare complication after tonsillectomy and mainly documented by case reports or a few retrospective and prospective trials with a limited number of patients. None could clarify frequency, time course, or prognosis of long-lasting dysgeusia after tonsillectomy. The aim of the study was to provide a symptom-based follow-up after tonsillectomy to assess postoperative taste disorders. STUDY DESIGN: Prospective clinical trial. METHODS: From December 2007 to June 2009 adult patients undergoing tonsillectomy were asked to take part in the trial. Two hundred twenty-three patients (119 female, 104 male; mean age, 33 ± 13 years) were included. The day prior to surgery, and 2 weeks and 6 months after tonsillectomy a standardized questionnaire was completed by patients. The questionnaire focused on taste function, taste disorders, pain, foreign body sensation, and bleeding episodes after tonsillectomy. RESULTS: One hundred eighty-eight (2 weeks) and 181 (6 months) patients returned the questionnaires. Thirty-two percent (n = 60) of patients reported taste disorders after tonsillectomy 2 weeks postoperatively and 15 patients (8%) at 6-month follow-up. Metallic and bitter parageusia were most frequently reported. The mean ratings of gustatory function were significantly lower 2 weeks after surgery (P < .001) and reached preoperative values 6 months after surgery. Almost 30% of patients reported postoperative bleeding, 10% long-lasting postoperative pain, and 20% foreign body sensation. CONCLUSIONS: Long-lasting taste disturbance (metallic and bitter parageusia) after tonsillectomy is more frequent than previously reported. Long-lasting pain and foreign body sensation seem to be common symptoms. With regard to these results, a thorough preoperative explanation is mandatory.

[Jugular and tympanic paragangliomas]. / Les paragangiomes jugulaires et tympaniques.

Jugular and tympanic paragangliomas are the most frequent tumors of the middle ear. They appear as a reddish, pulsatile, retrotympanic mass. Patients complain from hearing loss and pulsatile tinnitus. Tumors are most often benign. Secretory forms are rare. Angio-MRI and CT-scan are gold standard. MIBG or octreotid scintigraphies are helpful to rule out multiple lesions. The treatment consists in surgery alone, radiotherapy alone or association of both. Often, embolisation prior to surgery decreases hemorrhagic risks. Vital prognosis is good. The functional outcome is critical in extensive tumors.